Great prognosis of concurrent anti-GBM disease and IgA nephropathy in a young woman: A case report

Medicine (Baltimore). 2022 Sep 16;101(37):e30686. doi: 10.1097/MD.0000000000030686.

Abstract

Rationale: The causal relationship between anti-glomerular basement membrane (anti-GBM) disease and immunoglobulin A (IgA) nephropathy is still unclear and cases of concurrent anti-GBM disease and IgA nephropathy are very rare, especially with a good prognosis and long-term follow-up. Here, we report a case of concurrent anti-GBM disease and IgA nephropathy. By using corticosteroids and cyclophosphamide in combination with plasmapheresis, the patient achieved a very good prognosis with complete normalization of renal function and complete disappearance of hematuria and proteinuria at the subsequent follow-up. To our knowledge, no previous case with such a long follow-up and such a good prognosis have been reported.

Patient concerns: This case report describes a 26-year-old Chinese woman who presented with fever as the initial symptom, followed by dysmorphic hematuria, overt proteinuria and rapidly worsening renal function. Before admission, the patient received symptomatic supportive treatment such as intravenous albumin infusion, improvement of circulation, but the symptoms were not significantly improved.

Diagnosis: Per the results of kidney biopsy, the patient was diagnosed with crescentic glomerulonephritis and anti-GBM disease with IgA nephropathy.

Interventions: The key to obtain a good prognosis was the early application of corticosteroids and cyclophosphamide in combination with plasmapheresis to make the anti-GBM antibody turn negative quickly.

Outcomes: After 2 weeks of therapy, the patients' anti-GBM antibody turned negative and serum creatinine improved to a normal range. After 10 months, the patient's proteinuria level reached complete remission. After 12 months, the patient's hematuria had disappeared completely.

Lessons: This case provides experience in the treatment of concurrent anti-GBM disease and IgA nephropathy and highlights the importance of early application of plasmapheresis and immunosuppressive therapy to obtain a good prognosis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Albumins
  • Anti-Glomerular Basement Membrane Disease* / complications
  • Anti-Glomerular Basement Membrane Disease* / diagnosis
  • Anti-Glomerular Basement Membrane Disease* / therapy
  • Creatinine
  • Cyclophosphamide / therapeutic use
  • Female
  • Glomerulonephritis, IGA* / complications
  • Glomerulonephritis, IGA* / diagnosis
  • Glomerulonephritis, IGA* / therapy
  • Hematuria / etiology
  • Hematuria / therapy
  • Humans
  • Immunoglobulin A
  • Prognosis
  • Proteinuria / etiology
  • Proteinuria / therapy

Substances

  • Albumins
  • Immunoglobulin A
  • Cyclophosphamide
  • Creatinine